Publication

BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF

Journal Paper/Review - Mar 1, 2002

Units
PubMed

Citation
Schoch O, Schanz U, Koller M, Nakata K, Seymour J, Russi E, Boehler A. BAL findings in a patient with pulmonary alveolar proteinosis successfully treated with GM-CSF. Thorax 2002; 57:277-80.
Type
Journal Paper/Review (English)
Journal
Thorax 2002; 57
Publication Date
Mar 1, 2002
Issn Print
0040-6376
Pages
277-80
Brief description/objective

BACKGROUND: Idiopathic pulmonary alveolar proteinosis (PAP) has recently been recognised as a disease of impaired alveolar macrophage function caused by neutralising anti-granulocyte-macrophage colony-stimulating (anti-GM-CSF) autoantibodies. Subcutaneous recombinant human GM-CSF is a novel treatment for PAP, but its mechanism of action is unclear. METHODS: Clinical, functional, and bronchoalveolar lavage (BAL) findings were prospectively evaluated in a patient with PAP treated with daily subcutaneous GM-CSF 8 microg/kg for 12 weeks. RESULTS: Treatment resulted in improvements in dyspnoea, lung function, and peak cycle ergometry performance. In serum and BAL fluid the titre of anti-GM-CSF autoantibodies was raised at baseline and markedly reduced on treatment. At baseline the BAL fluid cellular profile showed a decrease in the absolute number and the percentage of macrophages (50%) and an increase in lymphocytes (45%), predominantly CD4+. This cellular distribution remained unchanged after 6 and 12 weeks of treatment while macrophages became morphologically normal and functionally improved. Extracellular proteinaceous material completely disappeared. CONCLUSIONS: Clinically successful treatment of PAP with GM-CSF was associated with a profound reduction in GM-CSF neutralising autoantibodies, improvement in alveolar macrophage morphology and function, but persistent BAL lymphocytosis.